Journal of Postgraduate Gynecology & Obstetrics is an Open Access, peer reviewed online journal published by Department of Obstetrics and Gynecology of Seth G. S. Medical College and K.E.M. Hospital, Parel, Mumbai, 400012, India.

Intestinal Malrotation In A Case Of Bicornuate Bicollis Uterus

Congenital malformations of
the female genital tract may be seen in approximately 5 to 7 percent of the
population. They are known to be associated with various other anomalies such
as renal, musculoskeletal, gastrointestinal etc. We report an unusual
association of an intestinal malrotation with the sigmoid colon on the right
side in a case of bicornuate bicollis uterus with a longitudinal vaginal
septum.

Introduction

Müllerian anomalies include
various malformations in the female genital tract. They may be associated with
renal and anorectal malformations, which occur sporadically, without any
familial tendency.[1] Of these anomalies, uterus didelphys due to a failure of
fusion of müllerian ducts, is seen in about 11%.[2] In 75% cases of didelphic
uterus a longitudinal vaginal septum is seen.[3] Renal anomalies are seen in
20% patients with uterus didelphys, with renal agenesis being the most common
anomaly. We report the case of a patient
uterus didelphys with intestinal malrotation.

Case Report

A 23 year old primigravida ,
married for 2 years, was scheduled for an elective cesarean section for breech
presentation at term. The breech presentation was persistent in her third
trimester. There was no medical or surgical illness in the past. There was no
history of any bowel or urinary bladder related symptoms. Her clinical
examination revealed a pulse of 86 beats per minute, blood pressure was
110/70mm of Hg. General and systemic examination revealed no abnormality.
Abdominal examination showed uterine size of 38 weeks, breech presentation, and
normal fetal heart sounds. A speculum examination showed complete longitudinal
vaginal septum and two cervices. A vaginal examination showed a complete
longitudinal vaginal septum and both the cervices uneffaced and closed.
Ultrasonography (USG) showed a bicornuate uterus, a single live intra uterine
gestation in the right horn of the uterus in breech presentation, and no
anomalies of maternal urinary tract. Investigations for fitness for anesthesia
showed normal findings. An elective lower segment caesarean section was
performed. The patient delivered a male child of 2.3 kg with APGAR 9/10.
Intraoperative findings revealed pregnancy in the right horn of a bicornuate
bicollis uterus. Incidentally it was also found that the sigmoid and the
appendix were on maternal right side. There was no intestinal obstruction. The
rest of the anatomy was normal. Post
operative period was uneventful and the patient made a complete recovery.

Figure 1. Intraoperative
findings: two horns of the uterus are seen from posterior aspect, the medial
end of the suture in the lower segment incision of the right horn is seen
(yellow arrow). The sigmoid colon is seen on the right side (black arrow).

Figure 2. Intraoperative
findings: two horns of the uterus are seen from posterior aspect, the appendix
is seen behind the right horn (black arrow).

Discussion

The uterus, fallopian tube,
cervix and upper two thirds of the vagina develop from a pair of mullerian
ducts while the lower third of the vagina develops separately from the
urogenital sinus. The co-existence of müllerian anomalies with renal anomalies
ipsilateral to the side of obstruction occurs due to arrest in embryological
development at the eighth week of gestation, which may affect both paramesonephric
and mesonephric ducts. Renal anomalies like ipsilateral renal agenesis, renal dysplasia, double collecting system and
ectopic ureter are known to be
associated with didelphys uterus.[4] It is also associated with
duplication of inferior vena cava, high-riding aortic bifurcation, malposition
of ovary and malrotation of intestine.[5] Uterus didelphys is a part of OHVIRA
syndrome (obstructed hemivagina, ipsilateral renal anomaly).[6] Malrotation may
occur alone or associated with other anomalies like atresia of small bowel.
During normal abdominal development, the gut herniates out from the abdominal
cavity, where it undergoes a 270 degrees counterclockwise rotation around the
superior mesenteric vessels. Following this rotation, the bowel returns to the
abdominal cavity, the duodenojejunal loop getting fixed to the left of the
midline and the cecum getting fixed in the right lower quadrant. Intestinal
nonrotation or incomplete rotation is any variation in this rotation and
fixation of the gastrointestinal tract during development. Intestinal rotation
and fixation can be interrupted during fetal development at different locations
leading to various presentations of disease.[5] Stage I of midgut rotation
occurs between 5-10 weeks' gestation, in which the physiologic herniation of
the bowel takes place. Nonrotation is the result of arrest in development at
stage I. As the gut lengthens without rotation, the mesentery forms a narrow
base predisposing to to volvulus. Stage II is at 10 weeks of gestation, in which the bowel returns to
the abdominal cavity. Incomplete rotation is due to arrest in stage II. It is
likely to result in duodenal obstruction by compression of its third portio, or
due to internal herniations. Stage III is from 11 weeks of gestation to term,
in which the cecum descends to the right lower quadrant and the mesenteries get
fixed.

This case is interesting
because of the association of malformations of the genital tract and
gastrointestinal tract. The incomplete rotation of the bowel made the patient
vulnerable to intestinal obstruction. She was counseled accoridngly and advised
to retain the operative notes carefully and report to a medical center in case
she developed constipation and vomiting associated with abdominal distension at
any time in future. No operative treatment was given for the incomplete
rotation because none was warranted.